Choroid plexus tumors of childhood

Roger J Packer MD

Neuro-Oncology

Updated 05.14.2024
Released 04.24.1995
Expires For CME 05.14.2027

Choroid plexus tumors of childhood

Author: Roger J Packer MD

See Contributor Disclosures

Choroid plexus tumors of childhood

In This Article

Quick Link

Introduction

Overview

Choroid plexus tumors of childhood are a relatively rare form of childhood brain cancer, comprising only 1% to 2% of all types of tumors that occur in patients younger than 18 years of age. Both choroid plexus papillomas and carcinomas can occur. Treatment of choice for both lesions is gross total resection. Patients with choroid plexus papillomas who have undergone complete resection rarely need alternative forms of adjuvant treatment. For patients with choroid plexus carcinomas, the need for adjuvant therapy following a gross total resection is unclear. Both radiotherapy and chemotherapy have shown questionable benefits for patients with subtotally resected choroid carcinomas. In this article, the author discusses some of the controversies in management and the genetic implications of a diagnosis of choroid plexus tumors.

Key points

	• Choroid plexus tumors, although relatively rare, do make up a significant component of central nervous system tumors occurring in the first year of life.
	• Separation of choroid plexus papillomas from atypical choroid plexus papillomas and atypical lesions from carcinomas is subjective, and the clinical course of atypical choroid plexus papillomas is less well characterized compared to other choroid tumor types.
	• Degree of surgical resection is likely the single most important factor in determining outcome for all types of choroid plexus tumors.

Historical note and terminology

Choroid plexus tumors of childhood are a rare form of childhood neoplasm, constituting no more than 2% of all childhood primary central nervous system tumors (48). Although other types of central nervous system tumors may arise in the choroid plexus, the vast majority of childhood choroid plexus tumors are either papillomas or carcinomas (26). In Kernohan and Fletcher-Kernohan's 1937 study of 109 cases of ependymoma, choroid plexus papillomas were classified as a subvariety of ependymoma (31). This type of classification was widely used as late as the 1970s. In the most recent World Health Organization classification of tumors of the central nervous system, choroid plexus tumors are considered a distinct variety of neuroepithelial tumor and are subclassified as choroid plexus papillomas (Gr I), atypical choroid plexus papillomas (Gr II), or choroid plexus carcinomas (Gr III). Papillomas that are well-differentiated are relatively easy to categorize; however, some papillomas have increased cellularity, nuclear pleomorphism, and increased mitotic activity and may be classified as atypical choroid plexus papillomas (41). The updated WHO classification of tumors of the central nervous system continues the same classification (42; 43).

Clinical manifestations

Presentation and course

Choroid plexus carcinomas vary significantly in size at the time of diagnosis. Usually, children with choroid plexus papillomas present with signs and symptoms of increased intracranial pressure, such as vomiting, headaches, and unsteadiness (27). Because up to 70% of choroid plexus tumors occur in children less than 2 years of age, with a median onset of 9 months, macrocephaly is often present, especially in congenital tumors (30; 29). In a series of 18 intracranial tumors diagnosed in the fetal stage, five were either choroid plexus carcinomas (two) or papillomas (three). Three had associated hydrocephalus (07). Given the chronicity of the tumor, it is somewhat surprising that patients usually have a relatively abrupt onset of symptoms before diagnosis, most commonly ranging from 1 week to 2 months. The hydrocephalus in patients with choroid plexus papillomas is believed to be secondary to the overproduction of cerebrospinal fluid (37; 18; 68). There may also be secondary hemorrhage within the cerebrospinal fluid and increased ventricular dilatation due to subarachnoid reabsorption block.

The clinical symptomatology of children with choroid plexus carcinomas differs somewhat from that of children with choroid plexus papillomas (40). Carcinomas tend to be more locally invasive. Children with choroid plexus carcinomas frequently have focal neurologic deficits without hydrocephalus early in the illness. Children younger than 3 years of age commonly present with vomiting and lethargy, although there is often associated hemiparesis. Older children are more likely to have focal neurologic deficits, which are later associated with headaches or vomiting, but no frank evidence of increased intracranial pressure until later in the illness.

Metastatic spread occurs more commonly in choroid plexus carcinomas than in papillomas. Although such dissemination may be symptomatic, in the majority of cases of disseminated choroid plexus carcinoma, dissemination is asymptomatic and is found on staging studies.

Clinical vignette

A 9-month-old child was brought to his pediatrician because of a 1-month history of vomiting. On examination, the child looked somewhat pale, was lethargic, and was mildly dehydrated. The child had a head circumference of 48 cm, which is well above the 98th percentile. Both the anterior and posterior fontanelles were open and the anterior fontanelle was somewhat full. On neurologic examination, the child was arousable but irritable. Pupils were equal and reactive and the discs were slightly pale. Extraocular movements were full, except for some limitation in upgaze. The remainder of the child’s cranial nerve examination was normal. On motor and coordination testing, there was mild to moderate hypotonia. The child, who was sitting by 6 months of age, could no longer sit. The child was supporting weight poorly. Reflexes were diffusely increased. On CT scan, a lobulated mass was found in the right lateral ventricle. The mass was hyperdense in comparison to the normal surrounding brain and enhanced homogeneously. There was marked associated hydrocephalus, with dilatation of both the lateral and third ventricle. On MRI, the mass appears papillary with relatively well-defined margins. At surgery a well-circumscribed choroid plexus papilloma was resected. The child had an intraventricular bleed following surgery and required 7 days of extraventricular drainage. The drain was removed and the hydrocephalus resolved without need for a ventricular peritoneal shunt.

Biological basis

Etiology and pathogenesis

Carcinomas may occur as part of the familial Li-Fraumeni syndrome, which is an autosomal dominant disorder caused by a germ-line mutation in the TP53 gene on chromosome 17p13 (45). Li-Fraumeni syndrome is a cancer predisposition syndrome manifest by germline mutations in the TP53 cancer suppressor gene. In one series of 42 children, six (16.7%) had phenotypic or genotypic characteristics consistent with Li-Fraumeni syndrome. Four of 11 with choroid plexus carcinomas were positive for TP53 germline mutations (21). Another series also found an association with TP53 germline mutations and choroid plexus carcinomas (19).

The choroid plexus papilloma is an epithelial tumor arising in the choroid plexus of the cerebral ventricles, composed of simple or pseudostratified layers of cuboidal or columnar cells, resting on a basement membrane, overlying papillary, vascularized connective tissue cores (58).

Choroid plexus papilloma (photomicrograph)

Choroid plexus papilloma are well-demarcated masses composed of delicate papillary fronds lining fibrovascular cores. The neoplastic cells rest on a continuous basement membrane and a connective tissue stroma. (Contributed by Dr. ...

It may be difficult to distinguish a papilloma from the more orderly normal choroid plexus, or from a papillary ependymoma on a small biopsy. Cytological atypia, occasional mitoses, and less commonly, foci of necrosis may be present. Demonstration of immunoreactivity to transthyretin may aid in the differential diagnosis. The World Health Organization classification recognizes an oncocytic variant (32). The classification makes no comment on malignant choroid plexus papillomas and uses the term "choroid plexus carcinoma" to designate a more malignant form of tumor. Choroid plexus papillomas are believed to cause ventricular dilatation due to overproduction of cerebrospinal fluid, although a partial block in cerebrospinal fluid reabsorption may also be present.

The choroid plexus carcinoma is a choroid plexus tumor with histological evidence of anaplasia, including increased mitotic activity, nuclear atypia, loss of papillary differentiation with transition to pattern-less cellular sheets, and necrosis (32).

Choroid plexus carcinoma (histology)

This hematoxylin and eosin preparation shows loss of papillary architecture and malignant cells. Elsewhere, there were large areas of necrosis. (Contributed by Dr. Sherman Stein.)

In adults, a rare variant may be difficult to distinguish from metastatic carcinoma to choroid plexus. Metastatic spread of nonprimary central nervous system tumors to the brain is rare in childhood.

The molecular biology of choroid plexus tumors is being elucidated. Transgenic mouse models have increased the understanding of choroid plexus carcinomas. The tumors were developed by activating the MYC oncogene and deleting the tumor TP53 suppressor gene in murine neural stem cells. These tumors resembled human choroid plexus carcinomas and exhibited multiple whole chromosomal losses (70).

Unique molecular signatures distinguish choroid plexus carcinomas from papillomas, including atypical choroid plexus papillomas; there are no clear molecular differences between papillomas and atypical papillomas (51). The majority of choroid plexus carcinomas harbor TP53 mutations, and those tumors that have two mutant copies are the most likely to be aggressive and result in poorer survival (51). Notch 3 signaling, the transcription factor TWIST1, platelet-derived growth factor receptor, and the tumor necrosis factor-related apoptosis-inducing ligand pathway have also been implicated in papilloma tumorigenesis (59). Molecular analysis has also shown that some choroid plexus carcinoma tumors, especially tumors of infancy, have been misdiagnosed as embryonal tumors (62).

Immunoreactivity for cytokeratin, vimentin, S-100 protein, transthyretin, and carboxyanhydrase, but not for epithelial membrane antigen, typifies choroid plexus carcinomas (16; 09; 20). The use of transthyretin as an immunohistochemical marker has been a significant advance in the diagnosis of choroid plexus tumors, especially in the distinction of primary choroid plexus tumors from other tumors that may invade the choroid plexus. Transthyretin is a transport protein for thyroxin and retinol. Transthyretin is believed, in theory, to be synthesized by the choroid plexus epithelium, and is, thus, useful as a marker for a primary choroid plexus neoplasm. In one review of 52 patients, the absence of transthyretin in tumor cells of patients with choroid plexus tumors was associated with a poorer prognosis (20). Other factors associated with a poorer prognosis included decreased immunoreactivity with the S100 protein, brain invasion by neoplastic cells, absence of stromal edema, and necrotic areas (04). The prognostic significance of atypical features of choroid plexus papillomas remains uncertain. In a series of 164 patients, elevated mitotic activity (more than two mitosis per 20 high-power field) was the sole feature independently associated with a higher likelihood of recurrence (28).

Methylation profiling has demonstrated three distinct subgroups: cluster 1 consisting of low risk tumors that contained primarily choroid plexus papillomas and atypical papillomas occurring in young children, primarily in the supratentorial space and having an excellent prognosis; cluster 2 arising in adulthood with predominant infratentorial origin and intermediate to excellent prognosis; and cluster 3, also found most commonly in the supratentorial space of young children, consisting of choroid plexus carcinomas, papillomas, and atypical papillomas. Cluster 3 may demonstrate a p53 abnormality, and those with p53 abnormalities have the poorest prognosis (65).

Furthermore, choroid plexus carcinomas clustered on DNA methylation profiles with homozygous TP53 mutations separately from those with heterozygous TP53 mutation or wild type TP53 and had the worst prognosis (56; 66).

In a proteogenomic characterization, choroid plexus tumors clustered in an “epithelial” cluster and demonstrated upregulation of immune-related pathways, as well as CTLA4 and PD-1 molecules (55).

Diagnostic workup

CT and MRI, which often may be complementary, are the primary components of the diagnostic workup of a child with a presumed choroid plexus carcinoma or papilloma. Neuroimaging of the entire craniospinal axis is indicated after detection for both tumors, although, the incidence of dissemination is much higher in children with choroid plexus carcinomas than in patients with papillomas (53).

CT and MRI are essentially the only tests needed to diagnose a choroid plexus tumor. Plain skull x-rays, although often abnormal, are nonspecific. Plain skull x-rays usually show spreading of the cranial sutures and an enlargement of the cranial vault. Presence of mottled calcifications within the ventricular pathway is highly suggestive of a papilloma, but is only seen in a minority of patients. CT studies of choroid plexus papillomas usually demonstrate well-marginated, smooth or lobulated masses filling the lateral ventricles (67). The mass is usually isodense or hyperdense, does not seem to invade the surrounding brain parenchyma, and tends to enhance homogeneously.

Choroid plexus carcinoma (unenhanced CT)

An isodense, lobulated tumor is seen within the right atrium. The necrotic center of the tumor is hypodense. There is hydrocephalus. (Contributed by Dr. Sherman Stein.)

There is usually significant hydrocephalus and there may or may not be peritumoral edema. On MRI, choroid plexus papillomas appear as papillary or cauliflower-like tumors with well-defined margins. On T1-weighted images, the tumor may be isointense, in comparison to brain parenchyma. The lesion tends to show marked contrast enhancement and there may be associated foci of necrosis or hemorrhage. Calcification is usually better appreciated on CT than on MRI.

Invasion of choroid plexus carcinomas into the surrounding brain is usually demonstrated by both CT and MRI. Peritumoral vasogenic edema is usually present. The typical appearance of a choroid plexus carcinoma on CT is that of an isodense to hyperdense heterogeneous mass. After contrast injection, the solid portion of the tumor usually shows intense homogeneous enhancement. Calcifications and cysts may be present. On MRI, choroid plexus carcinomas are typically hypointense on T1-weighted images. The solid portion of the tumor tends to enhance intensely and homogeneously with contrast. On T2-weighted images, the solid portion of the tumor may have associated cystic areas or necrotic regions. Although not essential for diagnosis, arteriography may be valuable as part of an intravascular embolization procedure to reduce blood flow to the tumor, prior to surgical excision.

Choroid plexus carcinoma (arteriogram)

Lateral view, arterial phase, internal carotid injection, showing an enlarged anterior choroidal artery (alongside needle on scalp) supplying the tumor. There is marked hydrocephalus. (Contributed by Dr. Sherman Stein.)

Because of the presence of hydrocephalus in patients with papillomas and increased intracranial pressure, due to local tumor invasion and associated vasogenic edema in patients with carcinomas, cerebrospinal fluid usually cannot be sampled prior to surgical intervention. In patients with both papillomas and carcinomas, cerebrospinal fluid protein will be elevated. Children with papillomas also frequently have hemorrhagic cerebrospinal fluid due to the vascular nature of the tumor. Cerebrospinal fluid is usually obtained following surgery in patients with carcinomas for evaluation of presymptomatic tumor spread.

Management

The management of choroid plexus papillomas is gross total surgical resection, when possible (22; 24; 23; 08; 52; 44). The complications of such resection have already been reviewed, including the possibility of death following surgery, due to tumor hemorrhage, hydrocephalus, or the development of postoperative subdural hematomas. Brain shift, secondary to acute CSF drainage, has been recognized a significant factor in morbidity (34). Preoperative embolization may allow safer, more extensive resection in larger tumors (61). There is no evidence that postoperative radiation improves survival of children after gross total resection. Radiotherapy has been used in children with subtotally resected choroid plexus papillomas, and some have used radiation following initial attempts at surgery, to shrink the tumor so that a second surgical resection could be attempted. The benefits of radiotherapy in treating subtotally resected tumors, or in shrinking disease prior to an attempt at surgical resection are poorly documented. The reported experience with chemotherapy is essentially nil.

Although choroid plexus carcinomas are malignant invasive tumors, surgery remains the primary modality of treatment (14; 54; 25). In various reviews, children with total surgical resections have fared much better than those with partial resections. In one review, 11 of 14 children had prolonged disease-free survival after gross total resections, only two of whom received adjuvant therapy. By contrast, only two of 20 children with choroid plexus carcinomas were reported to be alive and free of progressive disease with less than total resections, independent of the type of adjuvant therapy received.

Choroid plexus carcinoma (surgical specimen)

The lobulated, papillary, and vascular nature of the tumor can be seen. A gross total removal was accomplished. (Contributed by Dr. Sherman Stein.)

Although radiation therapy has been recommended for children with choroid plexus carcinomas after gross total resection, there is little proof of its benefit. In children with partially resected tumors, the benefits of radiation therapy are also questionable (03). There is some evidence that patients treated with radiotherapy after subtotal resections experience longer disease remissions, but there is no evidence that radiation therapy is curative (71; 72). Stereotactic radiosurgery has also been used with possible success (15).

The role of chemotherapy for choroid plexus carcinomas is far from settled (47; 01; 11; 71; 73; 35; 03). A variety of different chemotherapeutic agents have been used, including a 4-drug regimen of cyclophosphamide, vincristine, cisplatinum, and VP-16. A subgroup of infants treated with this regimen did well; however, the majority who fared well had gross total resection prior to the initiation of treatment (12; 08). A meta-analysis of case reports suggests that chemotherapy may be of some benefit (73); however, there is no consensus over what chemotherapy regimen is best. In a registry study of 35 children, retrospective analysis of nonrandomized patients suggested a carboplatin/etoposide regimen was superior, as regards to survival to cyclophosphamide and etoposide dual treatment (72). High-dose chemotherapy with autologous hematopoietic stem cell rescue has also been used with some benefit (74). Loss of TP53 expression may result in increased resistance to anticancer agents (33).

Because of the relatively high incidence of TP53 mutations and association of Li-Fraumeni syndrome, some have suggested that all patients with choroid plexus carcinomas be tested for germline mutations. In those testing positive, a possible approach is to screen all siblings, especially early in life, for development of intracranial tumors and to also perform more frequent screening in adult relatives for development of related malignancies such as breast, colon, and lung tumors (21). It has yet to be proven that earlier detection will improve overall outcome.

Outcomes

The overall 5-year prognosis for children with choroid plexus papillomas has improved. In early series, evaluating patients treated primarily before 1970, a surgical mortality rate as high as 30% was reported (69; 57; 02). Surgical mortality has fallen in series reviewing patients treated in the 1970s and 1980s (38). In a French series of 54 children with choroid plexus papillomas, 5-year survival was 100%; for 26 children with atypical papillomas, it was 96.2% (60). Because the majority of children with choroid plexus papillomas have significant hydrocephalus at the time of diagnosis, part of the surgical morbidity is associated with the development of subdural hematomas, secondary to collapse of the ventricular system after cerebrospinal fluid drainage or tumor removal. In addition, choroid plexus papillomas are highly vascular, and secondary hemorrhage within the brain after tumor removal may occur. In patients with gross total resections of choroid plexus papillomas who survive the postoperative period, overall survival is excellent. The vast majority of children who survive surgery and have a total removal of the tumor are cured of their lesion (44). A variety of surgical approaches have been used, including an open transcortical approach (36; 49). In a SEER registry review, with current treatment, over 95% of children with papillomas were found to be alive (10). The proliferative index of choroids plexus tumors may be somewhat predictive of outcome (04). In addition, methylation of the telomerase reverse transcriptase (TERT) promoter may portend poorer prognosis or transformation from a papilloma to a carcinoma (06). Stromal invasion does not signify a poorer prognosis (39). The quality of life of children after gross total resection is less than optimal, especially in extremely young children with choroid plexus papillomas. Complications include hydrocephalus with subdural collections, mental retardation, and motor impairments.

The prognosis of children with choroid plexus carcinomas is poorer than those of children with papillomas (05), with overall survival at 5 years of 65% in a series of 22 children (60). However, in this series, relapses were frequent, so event-free survival was only 25% at 5 years. The majority of reported children who have undergone partial resections have died of disease progression (54; 08; 71). In contradistinction, over two-thirds of children treated with gross total resections are alive and free of disease 5 years following treatment (14; 54; 17; 63; 10; 46; 25). Children with choroid plexus carcinomas may have significant residual neurologic compromise, especially with tumors that have invaded eloquent areas of the brain. These sequelae include hemiparesis, seizure disorders, and cognitive dysfunction.

Media

References

01: Allen JC, Wisoff J, Pierce J, Heston L. Choroid plexus carcinoma: responses to chemotherapy alone in newly diagnosed young children. Ann Neurol 1989;26:461.
02: Boyd MC, Steinbock P. Choroid plexus tumors: problems in diagnosis and management. J Neurosurg 1987;66:800-5. PMID 3572508
03: Cannon DM, Mohindra P, Gondi V, Kruser TJ, Kozak KR. Choroid plexus tumor epidemiology and outcomes: implications for surgical and radiotherapeutic management. J Neurooncol 2015;121(1):151-7. PMID 25270349
04: Carlotti CG Jr, Salhia B, Weitzman S, et al. Evaluation of proliferative index and cell cycle protein expression in choroids plexus tumors in children. Acta Neuropathol (Berl) 2002;103(1):1-10. PMID 11837741
05: Carpenter DB, Michelsen WJ, Hays AP. Carcinoma of the choroid plexus. J Neurosurg 1982;56:722-7. PMID 7069487
06: Castelo-Branco P, Choufani S, Mack S, et al. Methylation of the TERT promoter and risk stratification of childhood brain tumors: an integrative genomic and molecular study. Lancet Oncol 2013;14(6):534-42. PMID 23598174
07: Cavalheiro S, Moron AF, Hisaba W, Dastoli P, Silva NS. Fetal brain tumors. Childs Nerv Syst 2003;19(7-8):529-36. PMID 12908112
08: Chow E, Reardon DA, Shah AB, et al. Pediatric choroid plexus neoplasms. Int J Rad Oncol Biol Phys 1999;44:249-54. PMID 10760416
09: Coates TL, Hinshaw DB, Peckman N, et al. Pediatric choroid plexus neoplasms: MR, CT and pathologic correlation. Radiology 1989;173:81-8. PMID 2781035
10: Dudley RW, Torok MR, Gallegos D, Liu AK, Handler MH, Hankinson TC. Pediatric choroid plexus tumors: epidemiology, treatments, and outcome analysis on 202 children from the SEER database. J Neurooncol 2015;121(1):201-7. PMID 25297498
11: Duffner PK, Cohen ME, Horowitz M, et al. The treatment of choroid plexus carcinomas in infancy with chemotherapy. Ann Neurol 1989;26:460.
12: Duffner PK, Kun LE, Burger PC, et al. Postoperative chemotherapy and delayed radiation in infants and very young children with choroid plexus carcinomas. Pediatr Neurosurg 1995;22:189-96. PMID 7619719
13: Dunham C, Pillai S, Steinbok P. Infant brain tumors: a neuropathologic population-based institutional reappraisal. Hum Pathol 2012;43(10):1668-76. PMID 22497851
14: Ellenbogen RG, Winston KR, Krupsky WJ. Tumors of the choroid plexus in children. Neurosurgery 1989;25:327-35. PMID 2771002
15: Faramand A, Kano H, Niranjan A, et al. Stereotactic radiosurgery for choroid plexus tumors: Report of the International Radiosurgery Research Foundation. Neurosurgery 2021;88(4):791-6. PMID 33372216
16: Felix I, Phudhichareonrat S, Halliday WC, Becker LE. Choroid plexus tumors in children: immunohistochemical and scanning-electron-microscopic features. Pediat Neurosci 1987;13(5):263-9. PMID 2461551
17: Fitzpatrick LK, Aronson LJ, Cohen KJ. Is there a requirement for adjuvant therapy for choroids plexus carcinoma that has been completely resected. J Neuro-Oncol 2002;57:123-6. PMID 12125972
18: Ghatak NR, McWhorter JM. Ultrastructural evidence for CSF production by a choroid plexus papilloma. J Neurosurg 1976;45:409-15. PMID 956877
19: Giacomazzi J, Selistre SG, Rossi C, et al. Li-fraumeni and li-fraumeni-like syndrome among children diagnosed with pediatric brain cancer in southern Brazil. Cancer 2013;119(24):4341-9. PMID 24122735
20: Gianella-Borvadori A, Zeltzer PM, Bodey B, Nelson M, Britton H, Martin A. Choroid plexus tumors in childhood: response to chemotherapy and immunophenotypic profile using a panel of monoclonal antibodies. Cancer 1991;69(3):809-16. PMID 1370394
21: Gozali AE, Britt B, Shane L, et al. Choroid plexus tumors; management outcome, and association with the Li-Fraumeni syndrome: The Children’s Hospital Los Angeles (CHLA) experience. Pediatr Blood Cancer 2012;58:905-9. PMID 21990040
22: Gudeman SK, Sullivan HG, Rosner MH, Becker RP. Surgical removal of bilateral papillomas of the choroid plexus of the lateral ventricles with resolution of hydrocephalus. J Neurosurg 1979;50:677-81. PMID 430163
23: Guidetti B, Spallone A. The surgical treatment of choroid plexus papillomas: the results of 27 years experience. Neurosurg Rev 1981;4:129-37. PMID 7301142
24: Hawkins JC. Treatment of choroid plexus papillomas in children: a brief analysis of twenty years' experience. Neurosurgery 1980;6:380-4. PMID 7393420
25: Hosmann A, Hinker F, Dorfer C, et al. Management of choroid plexus tumors-an institutional experience. Acta Neurochir (Wien) 2019;161(4):745-54. PMID 30783805
26: Humphreys RP, Neomoto S, Hendrick EB, et al. Childhood choroid plexus tumors. In: Marlin AE, editor. Concepts in pediatric neurosurgery. Basel: Karger, 1984:1-18.
27: Humphreys RP, Neomoto S, Hendrick EB. Childhood choroid plexus tumors. In: Marlin AE, editor. Concepts in pediatric neurosurgery. Basel: Karger, 1987;7:1-18.
28: Jeibmann A, Hasselblatt M, Gerss J, et al. Prognostic implications of atypical histologic features in choroid plexus papilloma. J Neuropathol Exp Neurol 2006;65(11):1069-73. PMID 17086103
29: Johnson DL. Management of choroid plexus tumors in childhood. Pediatr Neurosci 1989;14:195-206. PMID 2485914
30: Jooma RD, Grant N. Third ventricle choroid plexus papillomas. Childs Brain 1983;10:242-50. PMID 6884126
31: Kernohan JW, Fletcher-Kernohan EM. Ependymomas, study of 109 cases. Res Publ Assoc Res Nerv Ment Dis 1937;16:182-209.
32: Kleihues P, Burger PC, Scheithauer BW, et al. Histological typing of tumours of the central nervous system. 2nd ed. Berlin: Springer-Verlag, 1993.
33: Krzyzankova M, Mertsch S, Koos B, et al. Loss of TP53 expression in immortalized choroid plexus epithelial cells results in increased resistance to anticancer agents. J Neurooncol 2012;109(3):449-55. PMID 22763759
34: Kumar R, Singh S. Childhood choroid plexus papillomas: operative complications. Childs Nerv Syst 2005;21(2):138-43. PMID 15309472
35: Lafay-Cousin L, Keene D, Carrett A-S. Choroid plexus tumors in children less than 36 months: The Canadian Pediatric Brain Tumor Consortium (CPBTC) experience. Childs Nerv Syst 2011;27:259-64. PMID 20809071
36: Lang SS, Beslow LA, Gabel B, et al. Surgical treatment of brain tumors in infants younger than six months of age and review of the literature. World Neurosurg 2012;78(1-2):137-44. PMID 22120270
37: Laurence KM. The biology of choroid plexus papilloma and carcinoma of the lateral ventricle. In: Vinken PJ, Bruyn GW, editors. Tumors of the brain and skull, part 2. New York: Elsevier, 1974:555-95.
38: Laws E, Hirsch JF. Choroid plexus papillomas: consideration of a surgical series of 33 cases. Acta Neurochir (Wien) 1996;138(7):810-6.
39: Levy ML, Goldfarb A, Hyder DJ, et al. Choroid plexus tumors in children: significance of stromal invasion. Neurosurgery 2001;48:303-9. PMID 11220372
40: Lewis R. Carcinoma of choroid plexus. Brain 1967;90:177-86. PMID 6023072
41: Louis DN, Ohgaki H, Wiestler OD, et al. The 2007 WHO classification of tumours of the central nervous system. Acta Neuropathol 2007;114(2):97. PMID 17618441
42: Louis DN, Perry A, Reifenberger G, et al. The 2016 World Health Organization classification of tumors of the central nervous system: a summary. Acta Neuropathol 2016;131(6):803-20. PMID 27157931
43: Louis DN, Perry A, Wesseling P, et al. The 2021 WHO Classification of Tumors of the Central Nervous System: a summary. Neuro Oncol 2021;23(8):1231-51. PMID 34185076
44: Lundar T, Due-Tønnessen BJ, Frič R, Brandal P, Due-Tønnessen P. Choroid plexus tumors in children: long-term follow-up of consecutive single-institutional series of 59 patients treated over a period of 8 decades (1939-2020). World Neurosurg 2022;158:e810-9. PMID 34823041
45: Malkin D, Li FP, Strong LC, et al. Germ line p53 mutations in a familial syndrome of breast cancer, sarcomas, and other neoplasms. Science 1990;250(4985):1233. PMID 1978757
46: Mallick S, Benson R, Melgandi W, Rath GK. Effect of surgery, adjuvant therapy, and other prognostic factors on choroid plexus carcinoma: a systematic review and individual patient data analysis. Int J Radiat Oncol Biol Phys 2017;99(5):1199-206. PMID 28939225
47: Maria BL, Graham ML, Strauss LC, Wharam MD. Response of a recurrent choroid plexus tumor to combination chemotherapy. J Neurooncol 1985;3:259-62. PMID 2414413
48: Matson DD, Crofton FD. Papilloma of the choroid plexus in childhood. J Neurosurg 1960;17:1002-27. PMID 13768200
49: Mazher S, Imran M, Ashraf J, Ahmed A, Shah IU, Zulfiqar F. Outcome of open transcortical approach in the management of intraventricular lesions. J Coll Physicians Surg Pak 2013;23(12):857-61. PMID 24304988
50: McEvoy AW, Harding BN, Phipps KP, et al. Management of choroid plexus tumours in children: 20 years experience at a single neurosurgical centre. Pediatr Neurosurgery 2000;321:192-9. PMID 10940770
51: Merino DM, Shlien A, Villani A, et al. Molecular characterization of choroid plexus tumors reveals novel clinically relevant subgroups. Clin Cancer Research 2015;21(1):184-92. PMID 25336695
52: Ogiwara H, Dipatri AJ Jr, Alden TD, Bowman RM, Tomita T. Choroid plexus tumors in pediatric patients. British J Neurosurg 2012;26(1):32-7. PMID 21970783
53: Packer RJ. Post-surgery management of children with brain tumors. In: McLaurin RL, Schut L, Venes JL, Epstein F, editors. Pediatric neurosurgery: surgery of the developing nervous system. 2nd ed. Philadelphia: WB Saunders, 1989:463-71.
54: Packer RJ, Perilongo G, Johnson DL, et al. Choroid plexus carcinomas of childhood. Cancer 1992;69:580-5. PMID 1728390
55: Petralia F, Tignor N, Reva B, et al. Integrated proteogenomic characterization across major histological types of pediatric brain cancer. Cell 2020;183(7):1962-85.e31. PMID 33242424
56: Pienkowska M, Choufani S, Turinsky AL, et al. DNA methylation signature is prognostic of choroid plexus tumor aggressiveness. Clin Epigenetics 2019;11(1):117. PMID 31409384
57: Raimondi AJ, Gutierrez FA. Diagnosis and surgical treatment of choroid plexus papillomas. Childs Brain 1975;1:81-115. PMID 1081032
58: Rubenstein LJ. Tumors of the central nervous system. Washington, DC: Armed Forces Institute of Pathology; 1972:85-104, 154-7, 259-62.
59: Safaee M, Oh MC, Bloch O, et al. Choroid plexus papillomas: advances in molecular biology and understanding of tumorigenesis. Neuro Oncol 2013;15(3):255-67. PMID 23172371
60: Siegfried A, Morin S, Munzer C, et al. A French retrospective study on clinical outcome in 102 choroid plexus tumors in children. J Neurooncol 2017;135(1):151-60. PMID 28677107
61: Slingerland AL, Papadakis JE, Staffa SJ, et al. Management of choroid plexus tumors and the benefit of preoperative embolization in pediatric patients: report of 46 cases from a single institution. World Neurosurg 2024;181:e1071-87. PMID 37977483
62: Sturm D, Orr BA, Toprak UH, et al. New brain tumor entities emerge from molecular classification of CNS-PNETs. Cell 2016;164(5):1060-72. PMID 26919435
63: Sun MZ, Ivan ME, Clark AJ, et al. Gross total resection improves overall survival in children with choroid plexus carcinoma. J Neurooncol 2014;116(1):179-85. PMID 24166581
64: Takaoka K, Cioffi G, Waite KA, et al. Incidence and survival of choroid plexus tumors in the United States. Neurooncol Pract 2022;10(1):41-9. PMID 36659972
65: Thomas C, Sill M, Ruland V, et al. Methylation profiling of choroid plexus tumors reveals 3 clinically distinct subgroups. Neuro Oncol 2016;18(6):790-6. PMID 26826203
66: Thomas C, Soschinski P, Zwaig M, et al. The genetic landscape of choroid plexus tumors in children and adults. Neuro Oncol 2021;23(4):650-60. PMID 33249490
67: Thompson JR, Harwood-Nash DC, Fitz CR. The neuroradiology of childhood choroid plexus neoplasms. Am J Roentgenol Radium Ther Nucl Med 1973;118(1):116-33. PMID 4540871
68: Tourcotte JF, Copty M, Bedard F, Michaud J, Verret S. Lateral ventricle choroid plexus papilloma and communicating hydrocephalus. Surg Neurol 1980;13:143-6. PMID 7355378
69: Turner OA, Simon MA. Malignant papillomas of choroid plexus: report of two cases with review of literature. Am J Cancer 1937;30:289-97.
70: Wang J, Merino DM, Light N, et al. Myc and loss of p53 cooperate to drive formation of choroid plexus carcinoma. Cancer Res 2019;79(9):2208-19. PMID 30885981
71: Wolff JE, Sajedi M, Brant R, Coppes MJ, Egeler RM. Choroid plexus tumours. Br J Cancer 2002;87:1086-91. PMID 12402146
72: Wolff JE, Van Gool SW, Kutluk T, et al. Final results of the choroid plexus tumor study CPT-SIOP-2000. J Neuroonco 2022;156(3):599-613. PMID 34997889
73: Wrede B, Liu P, Wolff JE. Chemotherapy improves the survival of patients with choroid plexus carcinoma: a meta-analysis of individual cases with choroid plexus tumors. J Neurooncol 2007;85(3):345-51. PMID 17576522
74: Zaky W, Dhall G, Khatua S, et al. Choroid plexus carcinoma in children: the Head Start experience. Pediatr Blood Cancer 2015;62(5):784-9. PMID 25662896

Patient Profile

Age range of presentation: 0 month to 12 years
Sex preponderance: male=female
Heredity: none
Population groups selectively affected: none selectively affected
Occupation groups selectively affected: none selectively affected

ICD & OMIM codes

ICD-10: Malignant neoplasm of cerebral ventricle: C71.5
ICD-11: Choroid plexus tumours: 2A00.22

Primary neoplasm of brain of unknown or unspecified type: 2A00.5

Other specified disorders of the nervous system: 8E4Y

Choroid plexus of fourth ventricle: XA1B86

Choroid plexus of lateral ventricle: XA1XM1

Choroid plexus of third ventricle: XA53A3

Choroid plexus: XA9KX2

Choroid plexus papilloma, NOS: XH0RF9

This is an article preview.
Start a Free Account
to access the full version.

Nearly 3,000 illustrations, including video clips of neurologic disorders.
Every article is reviewed by our esteemed Editorial Board for accuracy and currency.
Full spectrum of neurology in 1,200 comprehensive articles.
Listen to MedLink on the go with Audio versions of each article.

Questions or Comment?

MedLink®, LLC

3525 Del Mar Heights Rd, Ste 304
San Diego, CA 92130-2122

Toll Free (U.S. + Canada): 800-452-2400

US Number: +1-619-640-4660

Support: service@medlink.com

Editor: editor@medlink.com

ISSN: 2831-9125

Choroid plexus tumors of childhood

Differential Diagnosis

metastatic lesions
breast cancer
lung cancer
ependymomas
carcinomas
- papillomas
- hydrocephalus
- localized neurologic deficits
- increased intracranial pressure
- ependymoma
- childhood metastatic deposits
- immunohistochemical markers

Also Relevant

Atypical teratoid/rhabdoid tumors
Brain tumors in infancy and early childhood
Headache associated with intracranial neoplasms

Clinical Categories

Web References

Clinical Trials

NCI: Pediatric Oncology

Choroid plexus tumors of childhood

Choroid plexus tumors of childhood

Introduction

Overview

Key points

Historical note and terminology

Clinical manifestations

Presentation and course

Clinical vignette

Biological basis

Etiology and pathogenesis

Epidemiology

Prevention

Differential diagnosis

Diagnostic workup

Management

Outcomes

Special considerations

Anesthesia

Media

References

Contributors

Author

Patient Profile

ICD & OMIM codes

This is an article preview.
Start a Free Account
to access the full version.

Questions or Comment?

Also in This Category

NF2-related schwannomatosis

X-linked hydrocephalus (L1 syndrome)

Tumors of the skull base

Breath-holding spells

Cerebro-oculo-facio-skeletal syndrome

Acute cerebellar ataxia in children

Headache in children: overview and treatment approaches

Alternating hemiplegia of childhood

Choroid plexus tumors of childhood

Introduction

Overview

Key points

Historical note and terminology

Clinical manifestations

Presentation and course

Clinical vignette

Biological basis

Etiology and pathogenesis

Epidemiology

Prevention

Differential diagnosis

Diagnostic workup

Management

Outcomes

Special considerations

Anesthesia

Media

References

Contributors

Author

Patient Profile

ICD & OMIM codes

This is an article preview. Start a Free Account to access the full version.

Questions or Comment?

Also in This Category

NF2-related schwannomatosis

X-linked hydrocephalus (L1 syndrome)

Tumors of the skull base

Breath-holding spells

Cerebro-oculo-facio-skeletal syndrome

Acute cerebellar ataxia in children

Headache in children: overview and treatment approaches

Alternating hemiplegia of childhood

This is an article preview.
Start a Free Account
to access the full version.